A quality assessment based on these seven QIs is feasible. Overall, compliance rates were high; however, for BoM-3, the practice remains to be improved in some centers. Based on BoM-4 compliance rates, steroids are infrequently used concurrently with radiation therapy for malignant spinal cord compression. Extended fractionation for BoM was less frequently performed in academic than in nonacademic centers. The initiation of radiation therapy for brain metastases was more frequently delayed in academic than in nonacademic centers.

BACKGROUND: Severe hypertriglyceridemia (>1000 mg/dL) has a variety of causes and frequently leads to life-threating acute pancreatitis. However, the origins of this disorder are unclear for many patients. OBJECTIVE: We aimed to characterize the causes of and responses to therapy in rare cases of severe hypertriglyceridemia in a group of Japanese patients. METHODS: We enrolled 121 patients from a series of case studies that spanned 30 years. Subjects were divided into 3 groups: (1) primary (genetic causes); (2) secondary (acquired); and (3) disorders of uncertain causes. In the last group, we focused on 3 possible risks factors for hypertriglyceridemia: obesity, diabetes mellitus, and heavy alcohol intake. RESULTS: Group A (n = 20) included 13 patients with familial lipoprotein lipase deficiency, 3 patients with apolipoprotein CII deficiency, and other genetic disorders in the rest of the group. Group B patients (n = 15) had various metabolic and endocrine diseases. In Group C (uncertain causes; n = 86), there was conspicuous gender imbalance (79 males, 3 females) and most male subjects were heavy alcohol drinkers. In addition, 18 of 105 adult patients (17%) had histories of acute pancreatitis. CONCLUSION: The cause of severe hypertriglyceridemia is uncertain in many patients. In primary genetic forms of severe hypertriglyceridemia, genetic diversity between populations is unknown. In the acquired forms, we found fewer cases of estrogen-induced hypertriglyceridemia than in Western countries. In our clinical experience, the cause of most hypertriglyceridemia is uncertain. Our work suggests that genetic factors for plasma triglyceride sensitivity to alcohol should be explored. (C) 2017 National Lipid Association. All rights reserved.

Background: Numbers of patients with diabetic gangrene is increasing. Antimicrobial treatment is commonly used, however, limb amputation cannot be avoided in severe cases. For prophylaxis at operation, basic antimicrobial agents such as cefazolin are often administrated, however, severe infection could occur if resistant strains were cultured especially in immunosuppressive patients such as diabetes. The purpose of this study is to clarify bacterial species and their susceptibility to antimicrobial agents for patients of diabetic foot gangrene. Methods & Materials: Twenty-four patients (nine females) who had amputation on their legs for treating diabetic gangrene were enrolled from year 2002 to 2012. Among them, fifteen patients had diabetic history for over ten years, eleven patients were having repetitive hemodialysis. Their ages were 40–81(mean 67), average hospitalization period were 81.6 days. Results: As results, fifty-seven strains were isolated. Among them, 62% strains were Gram-positive cocci, 33% were Gram-negative rods, 5% were Gram-positive rods. Two or more strains were detected in fifteen patients. Indigenous bacteria of skin such as MSSA were most commonly cultured (n = 10). MRSA was found only in one patient. Sixty-seven percent of Peptostreptococci (n = 7), and seventy-five percent of E.coli (n = 4) were resistant to new quinolones. All Enterococci (n = 6) were susceptible to penicillin. Seventy percent of operated patients had no complications and discharged normally. Others had re-operation, including two cases dead due to heart disease. Most commonly used antimicrobial agents for prophylaxis were cefazolin (n = 8). However, sixty percent of all operated cases had resistant bacterial strain against cefazolin. Conclusion: We conclude that in order to avoid inappropriate anti-microbial therapy, it is important to confirm antimicrobial susceptibility with bacterial culture before operation. Use of cefazolin as the first choice prophylaxis antimicrobials should be re-considered for diabetic gangrene amputation.

Backgrounds: Familial apolipoprotein (apo) C-II deficiency is a very rare inherited disorder characterized by chylomicronemia. Since the discovery in 1978, reports on apo C-II deficient patients have been limited and only 13 different mutations in APOC2, a gene encoding apo C-II protein, were identified.Objectives: The objective is to investigate the biochemical and genetic features of a 3-month-old Bosniak girl with chylomicronemia whose apo C-II protein was undetectable in her plasma.Methods: APOC2, LPL, APOA5, and GPIHBP1 were sequenced. Isoelectrofocusing and irnmunoblotting of chylomicrons and VLDL fraction from the patient were performed.Results: Sequence analysis demonstrated a large deletion of 2978 base pairs in APOC2, which encompassed exons 2, 3, and 4. The patient was homozygous for the deletion. The 5' part of the breakpoint was located in an Alu Sx repetitive element in intron 1 of APOC2, whereas the 3' part of the breakpoint was in another Alu Sx between APOC2 and CLPTM1, a gene flanking APOC2. We speculate that the deletion was caused by a homologous recombination between two Alu Sx elements. No mutations were detected in LPL, APOA5, and GPIHBP1. Isoelectrofocusing and immunoblotting confirmed the absence of apo C-II protein.Conclusions: We diagnosed the patient as having apo C-II deficiency and designated the novel large deletion as apo C-IITuzla. This is the first description of apo C-II deficiency caused by Alu-Alu recombination in APOC2. (C) 2014 Elsevier B.V. All rights reserved.

Background Familial lipoprotein lipase (LPL) deficiency is a very rare autosomal recessive disorder characterized by marked elevation of plasma triglyceride concentrations. Since 1989, a variety of mutations have been reported in affected patients. Studies on subjects with heterozygous LPL deficiency, on the other hand, have been limited.Methods We examined post-heparin plasma LPL activity in 15 subjects with heterozygous LPL deficiency.Results The heterozygotes exhibited normal or slightly elevated plasma triglyceride concentrations. The mean LPL activity was reduced by 25% in the heterozygotes relative to controls. Interestingly, LPL activity was reduced specifically in female heterozygotes.Conclusion LPL activity is decreased in female, but not in male, subjects heterozygous for a number of different LPL gene mutations.

Background: The etiology of hypertriglyceridemia is complex and one of the common variants in affecting plasma lipid levels is apolipoprotein (apo) E isoform. Scores of apo E variants have been reported, including apo E7. However, a clinical lipid phenotype of apo E7 has not been fully elucidated.Methods: A 48-year-old Japanese male had hypertriglyceridemia and a history of repeated episodes of acute pancreatitis. The measurement of serum apolipoproteins and apo E phenotyping, and the sequencing analyses of several genes regulating triglyceride metabolism were performed in the patient.Results: The apo E phenotype of the patient was E7/E4.Apo E7 had the same point mutations p.[E244K; E245K] in APOE as reported previously. In addition, he had APOA5 haplotypes associated with hypertriglyceridemia. Laboratory examinations excluded deficiency of apolipoproteins, lipoprotein lipase, and GPI-HBP1 in this patient.Conclusions: This is, to our knowledge, the first report of severe hypertriglyceridemia and acute pancreatitis in a patient with apo E7. (C) 2014 Elsevier B.V. All rights reserved.

BACKGROUND:Type III hyperlipoproteinemia (HLP), a disorder associated with a high incidence of premature cardiovascular diseases, is characterized by the accumulation of remnant lipoproteins in the plasma. The primary genetic defect in patients with type III HLP is the presence of apolipoprotein E2 (apoE2), an isoform of apoE, and accumulation of remnant lipoproteins in the plasma has been thought to be attributable to the presence of apoE2, which bind poorly to low density lipoprotein receptors, resulting in defective remnant lipoprotein clearance. On the other hand, the activity of hepatic lipase (HL), the enzyme that plays a pivotal role in the removal of remnant lipoproteins, in type III HLP has not been investigated. METHODS:We examined post-heparin plasma lipoprotein lipase (LPL) and HL activities in 7 patients with type III HLP. The activities of HL and LPL in post-heparin plasma were measured separately by an immunochemical method using antiserum specifically directed against HL. RESULTS:The post-heparin plasma HL activity was significantly reduced, while the LPL activity was normal. CONCLUSIONS:Reduced HL activity may account, at least in part, for the accumulation of remnant lipoproteins in the plasma, a characteristic feature of type III HLP.

Desmoplastic small round cell tumor is a rare malignant tumor that occurs primarily in young males. Here, a case of small round cell tumor in an adult male successfully treated with a curative concurrent chemoradiotherapy is presented. A 58-year-old man had an intrapelvic tumor. Surgical resection was attempted, but the tumor was unresectable. Needle biopsy was performed and the diagnosis was suggested to be desmoplastic small round cell tumor. Concurrent chemoradiotherapy was performed, and a complete response was obtained. This patient has been alive for 8 years after treatment with no evidence of disease. Concurrent chemoradiotherapy appears to be a useful treatment choice for unresectable desmoplastic small round cell tumor. Iran. J. Radiat. Res., 2011; 9(3): 201­205

ApoA-V, ng/mL 16.4 23.0 179.2"74.8 Total cholesterol, mmol/L 1.76 3.81 -5.70 TG, mmol/L 1.61 5.10 -1.70 HDL-cholesterol, mmol/L 0.259 0.311 0.91–1.81 ApoA-I, g/L 0.304 0.335 0.89–1.92 ApoA-II, g/L 0.023 0.023 0.25–0.35 ApoB, g/L 0.331 0.381 0.04–1.28 ApoC-II, g/L 0.001 0.035 0.012–0.064 ApoC-III, g/L 0.026 0.119 0.034–0.126 ApoE, g/L 0.049 0.043 0.022–0.069 Reference range is expressed as mean"SD. Tetsu Ebara*, Hiroaki Hattori, Toshio Murase and Minoru Okubo 1 Okinaka Memorial Institute for Medical Research, Tokyo, Japan 2 Division of Advanced Technology and Development, BML Inc., Kawagoe, Japan 3 Department of Endocrinology and Metabolism, Toranomon Hospital, Tokyo, Japan

Background Aspirin esterase (AE) activity can account for part of aspirin pharmacokinetics in the circulation, possibly being associated with the impairment of aspirin effectiveness as an inhibitor of platelet aggregation. Aims The study was aimed at investigating the correlations of serum AE activity with cholinesterase (ChE) and metabolic variables in healthy subjects in comparison to subjects with type 2 diabetes mellitus (T2DM). Methods In cardiovascular disease-free T2DM subjects and healthy controls, the AE activity levels and/or the correlation patterns between AE and the other variables were analyzed. Results Neither AE nor ChE activities were higher in the subjects with T2DM. Serum AE activity strongly correlated with ChE as well as glucose/lipids variables such as total cholesterol and triglyceride in healthy subjects, while the correlations between AE and glucose/lipids variables were not present in T2DM subjects. Conclusions These data may reflect the pathophysiological changes between healthy and T2DM subjects. Our data may thus provide the basis for future studies to unravel the mechanisms.

The aim of this study is to validate the efficacy and feasibility of high-dose-rate brachytherapy (HDR-BT) combined with hypofractionated external beam radiotherapy (EBRT) for localized prostate cancer. Between December 2000 and December 2006, 278 patients with intermediate-risk or high-risk prostate cancer received a combination therapy of hypofractionated EBRT and HDR-BT. Hypofractionated EBRT using the three dimensional four-field technique was performed with a fractional dose of 3 Gy, three times a week, and a total dose of 51 Gy was delivered to the prostate and seminal vesicles but 45 Gy for patients treated with a fractional dose of 10.5 Gy in HDR-BT. After completion of EBRT, transrectal ultrasonography guided 192-iridium HDR-BT was performed. The fractionation of HDR-BT was as follows: 5 Gy x 5, 7 Gy x 3, 9 Gy x 2, or 10.5 Gy x 2 times, administered twice per day, and the biological effective dose for EBRT combined with HDR-BT, assuming that alpha-beta ratio is 3, was almost equal in each fractionation group. The median age of the patients was 65 years (range, 48-84), and the median initial PSA values were 12.9 ng/mL (range, 3.1-500 ng/mL). According to the T stage category, 61 tumors were judges as T1c, 55 as T2a-b, 41 as T2c, 96 as T3a, 25 as T3b. Centrally diagnosis Gleason score (GS) were as follows: GS5-6 in 26 patients, GS 7 in 91, GS 8-10 in 90. Consequently, 103 and 175 tumors were considered as intermediate-risk and high-risk prostate cancer, respectively. All patients received androgen deprivation therapy (ADT) consisting medical or surgical castration with or without anti-androgen. Neoadjuvant ADT was applied for 6-12 months for all patients, but adjuvant ADT was administered for 12 and 24 months for intermediate-risk and high-risk cancer patients, respectively. The median follow-up time was 57 months. The 5-year overall survival rate was 95%. Biochemical relapses were developed in 19 (7%), and clinical relapses were observed in 11 of them (prostate in 1, lymph nodes in 3, and bone in 7). The 5-year bNED rate was 90%, and T stage and GS were predictive factors for bNED. With regard to late toxicity, the Grade 2 and 3 genitourinary morbidities developed in 16 (5.8%) and 22 (7.9%) patients, respectively, and the Grade 2 and 3 rectal bleeding developed in 20 (7.2%) and 1 (0.4%) patients, respectively. HDR-BT combined with hypofractionated EBRT is an effective and feasible treatment method for intermediate- and high-risk prostate cancer patients, although further follow-up is necessary. To avoid occurrence of genitourinary and rectal complications, doses of the rectum and urethra in HDR-BT should be reduced.

AIM:To evaluate whether expression of L-type amino acid transporter 1 (LAT1) in pretreatment rectal cancer biopsies is predictive of tumour response to neoadjuvant hyperthermo-chemoradiotherapy (HCRT).PATIENTS AND METHODS:Forty-four patients with rectal adenocarcinoma who received neoadjuvant HCRT were investigated. LAT1 expression was immunohistochemically evaluated using pretreatment biopsies. The operation was performed after 2-3 months following HCRT and each resected specimen was graded by the histological criteria of the Japanese Classification of Colorectal Carcinoma.RESULTS:A positive LAT1 expression was recognized in 50.0% (22/44) of patients. Resected specimens were divided into 2 groups according to the histological grading criteria: good response (n=29) and poor response (n=15). LAT1-negative tumours had an 81.8% probability of good response and 18.2% probability of poor response. LAT1 expression showed marginally significant association with response to HCRT (p=0.05).CONCLUSION:LAT1 may be a useful predictive marker of response to HCRT in rectal cancer.

Yoshiko Aoyama, Yoriko Endo, Tetsu Ebara, Toshio Murase, Yoon S. Shin, Teodor Podskarbi, Isil Ozer, Mübeccel Demirkol, Gülden Gökçay and Minoru Okubo Okinaka Memorial Institute for Medical Research, Department of Endocrinology and Metabolism, Toranomon Hospital, Tokyo, Japan, Molecular Genetics and Metabolism Laboratory, Munich, Germany and Department of Pediatric Nutrition and Metabolism, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey